Proteus Syndrome in Intensive Care Unit: Case Report and Review of the Literature

Abstract

Proteus syndrome (PS) a complex disorder is rare with multisystem involvement. An extremely rare condition characterized by macrodactyly, vertebral abnormalities, asymmetric limb overgrowth and length discrepancy, hyperostosis, abnormal and asymmetric fat distribution, asymmetric muscle development, connective tissue nevi, and vascular malformations. It is progressive. Asymmetrical limb overgrowth is mostly seen and pathognomic. Patients have an unusual body habitus. The diagnosis may be delayed until late infancy because cutaneous lesions tend to appear over time, childhood, or even adulthood. We report a 16 years old boy who admitted to Intensive Care Unit (ICU) due to the arteriovenous malformation.